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Paper 4
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
A right-sided aortic arch with mirror imaging branching is the most common subtype of a right-sided aortic arch and is nearly always associated with congenital heart disease. Of these, 90% are associated with tetralogy of Fallot. Therefore other radiological findings to look for are a ‘boot-shaped’ heart and pulmonary oligaemia.
Oesophageal Disorders in Children
Published in John C Watkinson, Raymond W Clarke, Christopher P Aldren, Doris-Eva Bamiou, Raymond W Clarke, Richard M Irving, Haytham Kubba, Shakeel R Saeed, Paediatrics, The Ear, Skull Base, 2018
A comprehensive physical examination of the baby should be supplemented by a number of imaging modalities. An ultrasound scan of the kidneys is required to exclude associated renal anomalies. A cardiological assessment including echocardiography is essential pre-operatively to identify any life-threatening cardiac lesion and to locate the position of the aortic arch,3 which can be on the right side in 2% of cases. A right-sided aortic arch may make the surgery even more challenging than usual.
Congenital anomalies of the oesophagus
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
A right-sided aortic arch occurs in 5% of cases with OA. A right-sided aortic arch complicates exposure and repair of the oesophagus from the right side of the chest. Also, a right aortic arch is frequently associated with congenital heart disease or a vascular ring that completely encircles the trachea and oesophagus. When the aortic arch is on the right, the upper oesophageal pouch lies on the left side of the aortic arch, and the distal TOF lies to the left of the descending aorta. It is important to determine the position of the aorta preoperatively. An umbilical artery line noted on X-ray may indicate the side of the aortic arch. ECHO is useful for diagnosis but is operator dependent. CT angiography and MRI are the non-invasive imaging modalities of choice. Chest X-ray is not sensitive to diagnose a right-sided aortic arch.
Prenatal sonographic findings in a cohort of foetuses with a confirmed 22q11.2 microdeletion at a single Chinese Tertiary Centre
Published in Journal of Obstetrics and Gynaecology, 2022
Xiang-Yi Jing, Yong-Ling Zhang, Li Zhen, Yan-Lin Li, Dong-Zhi Li
In the study period, a total of 77 foetuses with del22q11.2 were detected by CMA. One case was excluded because of the loss to follow-up, and the remaining 76 cases were included in this study (shown in Figure 1). The median maternal age was 28 (19–39) years. The median gestational age at prenatal diagnosis was 25 (12–32) weeks. CHD was the most common indication (47/76, 61.8%) for prenatal testing and was isolated in 52.6% (40/76) (Table 1). The constitution of CHD comprised predominantly of conotruncal defects (61.7%; 29/47), in which tetralogy of Fallot (TOF) was the most common one (23.4%; 11/47), followed by interrupted aortic arch (14.9%; 7/47), truncus arteriosus (10.6%; 5/47), coarctation of aorta (6.4%; 3/47), pulmonary stenosis (4.3%; 2/47) and double-outlet right ventricle (DORV) (2.1%; 1/47), respectively. Other heart anomalies were encountered in 38.3% (18/47) of cases; among these, right-sided aortic arch (RAA) was found in 11 (23.4%; 11/47) cases, and as an isolated cardiac finding in 9 cases (19.1%; 9/47).
Deficiency of the Wnt receptor Ryk causes multiple cardiac and outflow tract defects
Published in Growth Factors, 2018
Kumudhini Kugathasan, Michael M. Halford, Peter G. Farlie, Damien Bates, Darrin P. Smith, You Fang Zhang, James P. Roy, Maria L. Macheda, Dong Zhang, James L. Wilkinson, Margaret L. Kirby, Donald F. Newgreen, Steven A. Stacker
Vascular corrosion casting of E18.5 embryos revealed the morphology of the great arteries and heart. Over 90% of Ryk−/− mice had defects in the aortic arch (Figure 2 and Table 1). Aortic arch stenosis (AAS; Figure 2(D,E,I–L)) or interruption of the aortic arch (IAA type-B, between the left common carotid (LCC) artery and the left subclavian artery (LSA); Figure 2(M–P)), were the most serious aortic arch defects observed. AAS and IAA were present in 53% and 13% of Ryk−/− mice, respectively (Table 1). Right-sided aortic arch (RSAA) occurred in 5% of Ryk−/− mice (Table 1; Figure 2(F–H)).
A late diagnosis of vascular ring: right-sided aortic arch with Kommerell diverticulum and aberrant left subclavian artery in an adolescent
Published in Acta Cardiologica, 2023
Jean-François Questiaux, Nesrine Farhat, Céline Kempeneers, Jaime F. Vazquez-Jimenez, Quentin Désiron, Pierre-Julien Bruyère, Jean-Olivier Defraigne, Marie-Christine Seghaye
Right-sided aortic arch with Kommerell diverticulum and aberrant left subclavian artery is a rare congenital malformation with an estimated prevalence of 0.5%. It results from the persistence of the right fourth branchial arch and incomplete regression of the fourth pharyngeal arch artery. The Kommerell diverticulum is the aneuvrysmatic origin of the aberrant left subclavian artery. Kommerell diverticulum, left aberrant retro-oesophageal subclavian artery and ligamentum arteriosum create a complete vascular ring that may compress oesophagus and trachea.